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|Year : 2017 | Volume
| Issue : 5 | Page : 1194-1195
A report of third case of Susac syndrome from India
Kamble J Harsha, Jeevan S Nair
Department of Clinical Neurosciences, Indo-American Hospital, Brain and Spine Centre, Chemmanakary, Kerala, India
|Date of Web Publication||6-Sep-2017|
Kamble J Harsha
Department of Clinical Neurosciences, Indo-American Hospital, Brain and Spine Centre, Chemmanakary - 686 143, Vaikom, Kerala
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Harsha KJ, Nair JS. A report of third case of Susac syndrome from India. Neurol India 2017;65:1194-5
A 26-year old male patient suffering from headache and left-sided hearing loss for the past one month presented with acute-onset giddiness, reduced verbal output, altered sensorium, vomiting, and giddiness. His magnetic resonance imaging (MRI) revealed typically asymmetric, multiple, discrete diffusion restricting, acute infarct-like lesions in the corpus callosum and bilateral cerebral white matter [Figure 1]a,[Figure 1]b,[Figure 1]c,[Figure 1]d,[Figure 1]e,[Figure 1]f. His blood and urine examinations were unremarkable. The cerebrospinal fluid examination showed no cells, mildly elevated protein, and normal sugar. The fundus examination showed multiple branch retinal artery occlusions, few Gass plaques within the retinal arteries, and a few hemorrhages with cotton-wool spots. Audiometry showed a left-sided, low frequency, sensory-neural hearing loss [Figure 1]g. As he was satisfying the criteria for Susac syndrome, he was managed with steroids and mycophenolate mofetil, following which his neurological status improved over a week's time.
|Figure 1: (a) FLAIR hyperintense lesions in corpus callosum showing diffusion restriction (b and c). (d) Sagittal T2 image showing perpendicular lesions in corpus callosum. (e) Few lesions also noted in cerebral white matter showing (f) diffusion restriction. (g) Pure tone audiometry showing normal hearing on right side ( marked with O and <), low frequency sensory neural hearing loss on left side (marked with X and ])|
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Susac syndrome (also known as SICRET [small infarctions of cochlear, retinal and encephalic tissue] syndrome) is a rare syndrome of unknown etiology causing microangiopathy of the retina, inner ear, and cerebral parenchyma. Less than 320 cases have been reported in the literature, while only two cases have been reported from India., Its clinical manifestations vary widely, from an initial hearing loss/visual loss to acute encephalopathy. The presence of an asymmetric hearing/visual loss is a rule rather than an exception; clinically missing these important clues is not uncommon. Acute involvement of the vestibular component is probably responsible for the acute-onset giddiness and vomiting in our case. The magnetic resonance imaging (MRI) appearance frequently mimics the imaging seen in multiple sclerosis/multiple embolic infarcts, hence delaying the appropriate diagnosis and treatment. A high index of suspicion while evaluating the MRI features along with the correlation with the findings on fundoscopic and audiometric examination help in clinching the diagnosis.
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